A 10-year-old girl presented with a 15-day history of excessive daytime sleepiness and sudden sleep onset, hypnagogic hallucinations, hyporexia, and behavioral changes. An MRI examination of the brain revealed a bilateral hypothalamic lesion (Figure). We found positive AQP4 -IgG antibodies in serum and low hypocretin levels (93 pg/mL) in the CSF. A diagnosis of narcolepsy secondary to neuromyelitis optica spectrum disorder was made. She improved after glucocorticoid administration. After 10 months of immunosuppressive maintenance therapy with azathioprine, she remains asymptomatic without new lesions in the follow-up neuroimages. Any diencephalic clinical syndrome, such as narcolepsy, with hypothalamic involvement, should prompt a serum test for AQP4 -IgG.1,2
Brain MRI after 2 weeks of symptoms, (A) Axial fluid-attenuated inversion recovery imaging and (B) coronal T2-weighted image demonstrated a bilateral hypothalamic hyperintense nonenhancing (C) lesion (arrows). (D) and (E) T2-weighted images show resolution of the lesion 3 months after diagnosis and treatment.
Study Funding
No targeted funding reported.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Appendix Authors
Footnotes
Go to Neurology.org/N for full disclosures.
↵* These authors contributed equally to this work.
Submitted and externally peer reviewed. The handling editor was Roy Strowd III, MD, Med, MS.
Teaching slides links.lww.com/WNL/C191
- Received January 14, 2022.
- Accepted in final form June 9, 2022.
- © 2022 American Academy of Neurology
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